Familial multiple endocrine neoplasia syndromes: components, classification, and nomenclature
Article first published online: 9 OCT 2008
Blackwell Science Ltd, 1997
Journal of Internal Medicine
Volume 243, Issue 6, pages 425–432, June 1998
How to Cite
Carney (1998), Familial multiple endocrine neoplasia syndromes: components, classification, and nomenclature. Journal of Internal Medicine, 243: 425–432. doi: 10.1046/j.1365-2796.1998.00345.x
- Issue published online: 9 OCT 2008
- Article first published online: 9 OCT 2008
- familial MEN syndromes;
- von Hippel–Lindau disease
Carney JA (Mayo Clinic and Mayo Foundation, Rochester, MN, USA). Familial multiple endocrine neoplasia syndromes: components, classification, and nomenclature (Minisymposium: MEN & VHL). J Intern Med 1998; 243: 425–32.
There are three generally accepted multiple endocrine neoplasia (MEN) syndromes: MEN-1, MEN-2A, and MEN-2B. On the basis of their major features, new familial MEN syndromes should manifest (i) a new endocrine tumour combination, (ii) autosomal dominant inheritance, and, possibly, (iii) a nonendocrine component. Acceptance of the last feature as a criterion has conferred candidate MEN status on a number of disorders, including von Hippel–Lindau disease and neurofibromatosis. To regard these conditions as MEN syndromes would require an inappropriate demotion of their predominant nonendocrine manifestations. The role of nonendocrine disorders in MEN syndromes needs reappraisal.